Rare surgery repairs child's damaged heart

Saturday

Mar 2, 2013 at 12:01 AMMar 28, 2013 at 10:29 AM

Cooper Ascone has spent the better part of the last two years getting picked, prodded and examined by doctors and nurses

TONY HOLTSTAFF WRITER

It pained Tommy Ascone to hear his son tell him his heart was hurting. Cooper, who turns 3 next month, wears a scar that runs vertically along his breastbone. Nowadays he does everything he couldn't do before his heart was fixed – including run, climb, play and eat a full meal. What matters most, his father said, is that Cooper looks happy. "Does your heart hurt now?" Tommy asked him. Cooper shook his head, sported a smile, pulled up his shirt for a moment to show his scar and ran back into the living room. He has spent the better part of the last two years getting picked, prodded and examined by doctors and nurses. Just as he seemed to be on the road to recovery, his symptoms would reappear and drag him down. Cooper has Shone's syndrome, a rare defect that disrupts the workings of two of the valves inside the heart. After all his checkups and all the conversations he heard about his diagnosis, Cooper began to pick up on what was happening. During a bad day, he would point to his heart and tell his mom and dad something was wrong. His father recalls those conversations and it still shakes him. "He's just too young to think like that," Tommy said. After a series of ineffective surgeries last year, Cooper's prognosis was turning bleak. One doctor told the boy's parents if something drastic couldn't be done for him, he would have only months to live. Luck, persistence and science saved Cooper. After six or seven doctors looked at test results, an astute resident cardiologist recognized the weak blood flow to Cooper's lower extremities. From that, Cooper was properly diagnosed. Cooper's mother, Jennifer, searched on Facebook and discovered a rare procedure to treat Shone's. It took perseverance for her to convince her husband such a procedure would work for their son. "I fought with her about it," Tommy admitted. "I told her we should just go with tried and true." In the end, the couple simply didn't like the other choices. One surgical option included inserting a bovine valve, which was too big for Cooper. Another option was to insert a mechanical valve, which was smaller but still probably too big. The latter also meant Cooper would need future open-heart surgeries and a lifelong reliance on blood-thinning medication. At first, those were the only choices being considered by doctors. It became Tommy's turn to do the urging – this time to Dr. Emile Bacha, a renowned pediatric heart surgeon. Tommy wanted Bacha to try something he had never done before. In the end, it worked. A transcatheter valve, known as a Melody valve, was inserted into Cooper's heart. The surgery was a success and Cooper's future is again boundless. His behavior has returned to normal. "It's night and day," Tommy said. "He's eating a lot more now. He's playing catchup."

RESIDENT WAS 'GUARDIAN ANGEL'

Shortly after Cooper's first birthday, his development halted. His twin sister, Chloe, was growing, walking, climbing the stairs and sleeping through the night while Cooper's potential to thrive at the same level had left him. She started to outgrow her brother. Cooper slept in spurts and would wake up in cold sweats. When he'd walk, he wouldn't take more than three steps before stopping and dropping to the floor. He frequently lost his appetite. Following their unsuccessful visits to doctors in Jacksonville and a battery of tests, a mistake led to the answer. One day, a nurse gave Cooper the wrong medication on a day he was scheduled to have another surgery. That led to a resident getting involved with Cooper's medical case. She had practiced medicine in Spain and was a physician-in-training at Wolfson Children's Hospital. The weak pulse in both of Cooper's feet was the first clue. The resident gave the boy a closer examination and she determined he wasn't getting the proper blood flow to his lower body. Shone's syndrome was the cause. By the time Cooper was properly diagnosed he was on the brink of heart failure, his parents said. "We really felt like she was his guardian angel," Tommy said. The couple never got the resident's name. Cooper wound up at New York-Presbyterian/Morgan Stanley Children's Hospital because Tommy and Jennifer wanted Bacha to handle their son's care. Cooper's condition is exceptionally rare, but Bacha's hospital admits up to five Shone's patients per year, making it one of the best-suited hospitals in the world for treatment of the disease. The couple's next-door neighbor, who works for a company that manufactures pieces for heart valve replacements, also assured them that Bacha was one of the best surgeons in the world in the field of pediatric cardiology, Tommy said. Bacha's first surgery on Cooper was in January. He repaired one of the damaged valves, but it started leaking soon after the surgery. "At that point, it was clear he needed a valve replacement," Bacha said. Within a few months, Cooper lay in a New York hospital bed in a medically induced coma with a variety of tubes and intravenous therapy needles sticking out of him, but the breakthrough surgery was successful.

NOW COOPER'S 'HAPPY, TOO'

Jennifer learned about the Melody valve surgery on a Facebook page for mothers with children stricken with Shone's syndrome. It had been used for other heart conditions, but hardly ever for a Shone's patient. "At first I said to them, 'You don't want your son to be a guinea pig,' " recalled Bacha. There was only one surgeon in the country that had inserted a Melody valve into a Shone's child prior to Cooper's surgery last month. It happened to be one of Bacha's former students, who was practicing medicine in Boston. He had performed the surgery about a half-dozen times. The two of them consulted and Bacha's own heart changed. He agreed to do the surgery. "The lesson really was that you can be a big-shot surgeon, but sometimes a patient's parents can come to you with great ideas for their kids," Bacha said. "That's why you have to keep an open mind. In this case, it radically changed the quality of life for their son." Tommy and Jennifer assumed Bacha wasn't going forward with the Melody valve procedure until the morning of Cooper's surgery. They were at New York Presbyterian waiting on a meeting with Bacha when he walked in holding the valve in his hand. "He pulled out the valve and I was like . . ." said Jennifer, who re-enacted her own open-mouthed shocked expression. The Melody valve was successfully inserted into Cooper's heart. It can dilate over time and not have to be replaced through more open-heart surgeries. Cooper was diagnosed with Shone's in October 2011. It took 16 months of highs and lows, overnight trips to Jacksonville and New York, flirtations with using a valve from a cow's heart and tens of thousands of dollars not covered by insurance before Cooper's condition improved to the point the Ascone family could have peace of mind again. It was a happy ending, but still not entirely satisfying. Cooper will still have medical procedures in his future – albeit less dramatic. Additionally, Tommy and Jennifer Ascone, who added a third child to their family more than a year ago, face exorbitant medical expenses. News reports last year of fundraisers for the Ascone family came with skepticism from the public, the couple said. Tommy is a lieutenant with the Palm Coast Fire Department and Jennifer is a licensed chiropractor who owns a 50 percent share of a successful business. People wondered why they were trying to raise money when they were gainfully employed and insured. Their insurance covered much of Cooper's expenses, but the family still needed help with day care and traveling. They missed work to spend several nights with their son, who remained intubated and unconscious while in the hospital in New York. Community fundraisers got them to New York and for that they're appreciative. They are still waiting to see what medical charges will be written off and what will ultimately be billed to them. They expect to be close to $20,000 in debt. For now, they're enjoying being home with three healthy children. A couple of weeks ago, Tommy stood over his son, who was awake and recovering in his hospital bed. Cooper noticed the stressful look on his dad's face had been replaced. "Daddy, are you happy?" the boy asked. "Yes, I'm happy," his dad told him. "Then I'm happy, too."

Editor's note: The surgery was performed at New York-Presbyterian/Morgan Stanley Children's Hospital, and not as originally reported.